PATHOGENIC AND ADAPTIVE MOLECULAR INTERACTIONS WITH MUTANT HUNTINGTIN EXON 1

Nascent mutant Huntingtin exon 1 chains do not stall on ribosomes during translation but aggregates do recruit machinery involved in ribosome quality control and RNA

Angelique R Ormsby, Dezerae Cox, James Daly, David Priest, Elizabeth Hinde, Danny M Hatters

2020-07-31

Mutations that cause Huntington's Disease involve a polyglutamine (polyQ) sequence expansion beyond 35 repeats in exon 1 of Huntin..

A thiol probe for measuring unfolded protein load and proteostasis in cells

Moore Z Chen, Nagaraj S Moily, Jessica L Bridgford, Rebecca J Wood, Mona Radwan, Trevor A Smith, Zhegang Song, Ben Zhong Tang, Leann Tilley, Xiaohong Xu, Gavin E Reid, Mahmoud A Pouladi, Yuning Hong, Danny M Hatters

2017-09-07

When proteostasis becomes unbalanced, unfolded proteins can accumulate and aggregate. Here we report that the dye, tetraphenylethe..

Transcriptional profiles for distinct aggregation states of mutant Huntingtin exon 1 protein unmask new Huntington's disease pathways

Nagaraj S Moily, Angelique R Ormsby, Aleksandar Stojilovic, Yasmin M Ramdzan, Jeannine Diesch, Ross D Hannan, Michelle S Zajac, Anthony J Hannan, Alicia Oshlack, Danny M Hatters

2017-09-01

Huntington's disease is caused by polyglutamine (polyQ)-expansion mutations in the CAG tandem repeat of the Huntingtin gene. The c..

Conformational dynamics and self-association of intrinsically disordered Huntingtin exon 1 in cells

Steffen Buening, Abhishek Sharma, Shivang Vachharajani, Estella Newcombe, Angelique Ormsby, Mimi Gao, David Gnutt, Tobias Voepel, Danny M Hatters, Simon Ebbinghaus

2017-05-07

Huntington's disease is caused by a CAG trinucleotide expansion mutation in the Huntingtin gene that leads to an artificially long..

Huntingtin Inclusions Trigger Cellular Quiescence, Deactivate Apoptosis, and Lead to Delayed Necrosis

Yasmin M Ramdzan, Mikhail M Trubetskov, Angelique R Ormsby, Estella A Newcombe, Xiaojing Sui, Mark J Tobin, Marie N Bongiovanni, Sally L Gras, Grant Dewson, Jason ML Miller, Steven Finkbeiner, Nagaraj S Moily, Jonathan Niclis, Clare L Parish, Anthony W Purcell, Michael J Baker, Jacqueline A Wilce, Saboora Waris, Diana Stojanovski, Till Bocking

2017-05-02

Competing models exist in the literature for the relationship between mutant Huntingtin exon 1 (Httex1) inclusion formation and to..

When Proteostasis Goes Bad: Protein Aggregation in the Cell

Mona Radwan, Rebecca J Wood, Xiaojing Sui, Danny M Hatters

2017-02-01

Protein aggregation is a hallmark of the major neurodegenerative diseases including Alzheimer's, Parkinson's, Huntington's and mot..

N-Terminal Fragments of Huntingtin Longer than Residue 170 form Visible Aggregates Independently to Polyglutamine Expansion

Moore Z Chen, Sue-Ann Mok, Angelique R Ormsby, Paul J Muchowski, Danny M Hatters

2017-01-01

BACKGROUND: A hallmark of Huntington's disease is the progressive aggregation of full length and N-terminal fragments of polygluta..